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Denise Ney PhD, University of California, Davis Affiliate Faculty Professor of Nutritional Sciences Contact Information: Waisman Center UW-Madison 1500 Highland Avenue Madison, WI 53705 ney@nutrisci.wisc.edu http://www.nutrisci.wisc.edu/fac_ns/f_ney.html |
Nutritional management of phenylketonuria.
Ney DM, Draxler AK, van Calcar SC, Gleason ST, Nelson KT, Lim K, Etzel MR.
Efficacy of Glycomacropeptide in the Nutritional Management of Phenylketonuria.
Conference Proceedings, International Whey Conference, 2005, Chicago, IL.
Last updated 4/5/2006 by rowley@waisman.wisc.edu
Our research group is conducting a clinical study in subjects with
phenylketonuria (PKU) to determine the safety and efficacy of glycomacropeptide
from cheese whey in the nutritional management PKU. Individuals with PKU have a
deficiency of the liver enzyme phenylalanine hydroxylase which converts the
essential amino acid phenylalanine to tyrosine. To prevent brain damage and
cognitive impairment they must follow a lifelong, low-phenylalanine diet that is
restricted in natural protein and requires ingestion of a bitter tasting amino
acid formula. Glycomacropeptide is uniquely suited to the PKU diet as it is the
only known dietary protein that is naturally free of phenylalanine.
Low-phenylalanine foods and a palatable alternative to the amino acid
formulation can be made from glycomacropeptide.
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Ney